Abstract

Background:

Childhood interstitial lung disease (chILD) is a heterogeneous group of rare and severe diseases. Epidemiological data are limited and chILD is most likely under-diagnosed. As with all rare diseases, the establishment of networks of expertise and national or international databases are crucial for increasing chILD knowledge. The first multidisciplinary team meetings (MDTm) dedicated to chILD have been set up in frame of the RespiRare network in France since 2018. We aim to study the contribution of MDTm in the diagnosis and management of chILD.

Methods:

We conducted a retrospective and descriptive study on the chILD MDTm reports from 2018 to 2022. Each meeting was attended by a quorum and by pediatric pulmonologists, radiologists, geneticists and pulmonologists (mean 13 participants). They lasted for 2 hours and were held monthly via video conference. A written report followed the meeting.

Results:

178 chILD cases were discussed (45% females). The median age of onset was 0.5 years [IQR 0;7]. The MDTm allowed to rectify the chILD etiology for 33% (NEHI 20.2%, surfactant disorders 8.4%, sarcoidosis 2.8%, autoimmune chILD 1.6%, other chILD 41%, undefined chILD 19.6%) and to exclude chILD for 6.2%. A therapeutic change was proposed in 43%.

Discussion and conclusion:

Our experience shows that a dedicated MDTm provides a unique opportunity to improve chILD etiologic diagnosis and to adapt management and therapy in chILD.